Risk Factors of Early Mortality and Morbidity in Esophageal Atresia with Distal Tracheoesophageal Fistula: A Population-Based Cohort Study.

OBJECTIVE: To identify the risk factors for early mortality and morbidity in a population with distal esophageal atresia (EA)-tracheoesophageal fistula. STUDY DESIGN: Cohort study from a national register. Main outcomes and measures included early mortality, hospital length of stay (LoS), need for nutritional support at 1 year of age as a proxy measure of morbidity, and complications during the first year of life. RESULTS: In total, 1008 patients with a lower esophageal fistula were included from January 1, 2008, to December 31, 2014. The survival rate at 3 months was 94.9%. The cumulative hospital LoS was 31.0 (17.0-64.0) days. Multivariate analysis showed that intrahospital mortality at 3 months was associated with low birth weight (OR 0.52, 95% CI [0.38-0.72], P < .001), associated cardiac abnormalities (OR 6.09 [1.96-18.89], P = .002), and prenatal diagnosis (OR 2.96 [1.08-8.08], P = .034). LoS was associated with low birth weight (-0.225 ± 0.035, P < .001), associated malformations (0.082 ± 0.118, P < .001), surgical difficulties (0.270 ± 0.107, P < .001), and complications (0.535 ± 0.099, P < .001) during the first year of life. Predictive factors for dependency on nutrition support at 1 year of age were complications before 1 year (OR 3.28 [1.23-8.76], P < .02) and initial hospital LoS (OR 1.96 [1.15-3.33], P < .01). CONCLUSIONS: EA has a low rate of early mortality, but morbidity is high during the first year of life. Identifying factors associated with morbidity may help to improve neonatal care of this population.

Esophageal atresia and tracheoesophageal fistula associated with tetralogy of Fallot: a review of mortality.

INTRODUCTION: Oesophageal atresia ± tracheoesophageal fistula (EA/TEF) associated with congenital heart disease (CHD) carries a worse prognosis than EA/TEF alone. Though the Spitz classification takes major CHD into account, there are no data regarding survival with the specific combination of EA/TEF and Tetralogy of Fallot (TOF). With advances in postnatal care, we hypothesised that, survival is improving in these complex patients. This study reports morbidity and mortality outcomes of newborns with oesophageal atresia and TOF cardiac malformations METHODS: All patients with EA/TEF and TOF treated at Alder Hey Children's Hospital between the years 2000-2020, were identified. Data sets regarding gestation, birth weight, associated anomalies, operative intervention, morbidity, and mortality were analysed. RESULTS: Of a total of 350, EA/TEF patients 9 (2.6%) cases had EA/TEF associated with TOF (M:F 4:5). The median gestational age was 35/40 (range 28-41 weeks) with a median birth weight of 1790 g (range 1060-3350 g). Overall survival was 56% (5/9 cases) and all survivors remain under follow up (range 37-4458 days). Surgical strategies for managing EA/TEF with Fallot's tetralogy included 6/9 primary repairs and 3/9 cases with TEF ligation only (+ gastrostomy ± oesophagostomy). CONCLUSIONS: This study reports outcome data from one of the largest series of EA TEF patients with Fallot's tetralogy. Whilst outcomes may be challenging for this unique patient cohort, survival metrics provide important prognostic information that can be widely shared with health care teams and parents.

Potential risk factors and outcomes of fistulas between the upper intestinal tract and the airway following Ivor-Lewis esophagectomy.

Fistulas between the upper intestinal tract and the airway following esophagectomy are a rare and severe complication with significant mortality. Treatment and therapy are difficult and require a multidisciplinary approach. The objective of this retrospective study was to identify risk factors for these fistulas following esophagetcomy, and to assess their impact on the further clinical course and outcome. 211 patients undergoing Ivor-Lewis esophagectomy for esophageal cancer between 2005 and 2012 were included. The preoperative risk factors including the risk score according to Schröder et al. and the O-Physiological and Operative Severity Score (POSSUM) score, operative and postoperative parameters and the outcome were evaluated. 65% of all patients developed postoperative complications, including 12 patients that developed fistulas between the upper intestinal tract and the airway (airway fistulas [AF]; 5.6%). Neither patient related risk factors nor esophagus-specific risk scores correlated with occurrence of AF. Furthermore, surgical treatment and neoadjuvant treatment did not show any effect on development of AF in our patients. However, we could demonstrate that AF significantly impacted on length of hospital stay (AF 52 days vs. No-AF group 16 days, P < 0.001), incidence of major pulmonary complications (83.3% vs. 17.1%, P < 0.001), 90-day mortality (42% vs. 7.5%, P = 0.002) and overall survival (133 days vs. 636 days, P=0.029). With the current study, we could not identify any patient related risk factors, esophagus-specific risk scores or treatment related details that might be useful as predictors of AF after Ivor-Lewis esophagectomy. However, we confirmed that AF significantly impacted on outcomes. This highlights the urgent need for further studies on this rare but devastating complication after esophagectomy.

Postintubation Tracheoesophageal Fistula - Diagnosis, Treatment and Prognosis.

Introduction: Postintubation tracheoesophageal fistula is a severe complication occurring under certain conditions in patients that require prolonged mechanical ventilation. MATERIAL AND METHODS: This article focuses on a sample of 11 patients with postintubation tracheoesophageal fistula, operated in our department between 2005 and 2015. The anterior approach with tracheal resection was performed in 10 of these patients, while an atypical surgical technique was preferred in a case involving a large-sized fistula. Three of these patients were subject to surgery while still on the ventilator, in order to help weaning them from mechanical ventilation. Two patients were operated following a relapse of the fistula, after attempts of closing it in other surgical units. Results: Two patients (of those who were still on mechanical ventilation) died from intubation-related complications that persisted after tracheal resection (anastomotic dehiscence with mediastinitis and tracheoarterial fistula in the brachiocephalic arterial trunk). The nine remaining patients improved, with their airways restored and having regained normal deglutition. Conclusions: The surgical approach of this pathology is successful in surgical units that are specialised in tracheal and oesophageal surgery. Adequately timing the surgery is crucial for a good outcome.

Performance of prenatal diagnosis in esophageal atresia.

OBJECTIVE: The aim of this study was to evaluate the performance of prenatal diagnosis of esophageal atresia (EA) and its associated abnormalities. METHODS: We conducted a retrospective study from a pediatric database of EA managed postnatally in a single center. Prenatal data included ultrasound and magnetic resonance imaging parameters including amniotic fluid (AF) volume, stomach visualization, AF biochemistry, and associated malformations. Postnatal data included type of EA, mortality, and postnatal diagnosis of associated malformations. RESULTS: One hundred twenty-two cases were included. The diagnosis was suspected prenatally in 39/122 (32%) cases. Polyhydramnios was noted in 64/122 (52.4%), and the stomach was not visualized or small in 39 (32%). There was 14 (11.5%), 2 (1.6%), 101 (82.8%), 5 (4.1%), and 0 (0%) types I, II, III, IV, and V, respectively. EA was suspected prenatally in 12/14 (85.7%) in type I and in 27/108 (25%) in cases with tracheoesophageal fistula (II + III + IV + V). Magnetic resonance imaging was performed in 28 cases, which confirmed EA in 19/28 (sensitivity 67.8%). AF biochemistry was performed in 17 cases, which confirmed EA in 15/17 (sensitivity 88.2%) cases. Of the 69 syndromic associations, 41/69 (59.4%) cases were detected prenatally. Associated malformation was a strong predictor of postnatal death [19/69 vs 3/53, odds ratio 6.33 (1.76; 22.75), p < 0.01]. CONCLUSION: Prenatal diagnosis of EA remains challenging. MRI and AF biochemistry may prove useful in the diagnosis of EA. Prenatal ultrasound and MRI examination should also focus on associated anomalies. © 2015 John Wiley & Sons, Ltd.

Esophageal atresia: a critical review of management at a single center in Algeria.

The purpose was to study the outcomes and factors affecting the survival of esophageal atresia in our center. A retrospective analysis of 86 cases of esophageal atresia (EA) over a 10-year period was performed with 46 boys and 42 girls. Demographic data, birth weight, gestational age, consanguinity, incidence of associated anomalies, place of delivery, history of feeding, and outcomes were studied. EA with distal tracheoesophageal fistula (TEF) was the commonest type with 58/86 (67%). The percentage of patients with at least one associated anomaly was 52/86 (60%), with 7/86 (8%) who are from consanguineous parents; most commonly associated anomalies were cardiac 13/86 (15%). The average gestational age and birth weight were 36 ± 2 weeks and 2300 ± 570 g, respectively. Survival rates for the patients according to the Waterston classification was 80% in group A, 58% in group B, and 25% in group C (three patients died before surgery). Prematurity, the gap between the two ends of the esophagus, and preoperative respiratory status were the most significant factors affecting the survival. Late complication of EA/TEF include respiratory symptoms, especially in the first year, associating tracheomalacia and bronchopulmonary infections in about 24/45 (53%), recurrence of TEF 3/45 (7%), esophageal stricture 26/45 (58%), and gastroesophageal reflux 22/45 (49%). The high incidence of delayed diagnosis, low birth weight, and lack of advanced neonatological management are important contributory factors to the poor outcome. The frequency of late complications highlights the need for multidisciplinary clinics to follow these children's.

A nationwide analysis of clinical outcomes among newborns with esophageal atresia and tracheoesophageal fistulas in the United States.

BACKGROUND: The aim of this study was to examine national outcomes in newborn patients with esophageal atresia and tracheoesophageal fistula (EA/TEF) in the United Sates. METHODS: Kids' Inpatient Database (KID) is designed to identify, track, and analyze national outcomes for hospitalized children in the United States. Inpatient admissions for pediatric patients with EA/TEF for kids' Inpatient Database years 2000, 2003, 2006, and 2009 were analyzed. Patient demographics, socioeconomic measures, disposition, survival and surgical procedures performed were analyzed using standard statistical methods. RESULTS: A total of 4168 cases were identified with diagnosis of EA/TEF. The overall in-hospital mortality was 9%. Univariate analysis revealed lower survival in patients with associated acute respiratory distress syndrome, ventricular septal defect (VSD), birth weight (BW) < 1500 g, gestational age (GA), time of operation within 24 h of admission, coexisting renal anomaly, imperforate anus, African American race, and lowest economic status. Multivariate logistic regression identified BW < 1500 g (odds ratio [OR] = 4.5, P < 0.001), operation within 24 h (OR = 6.9, P < 0.001), GA <28 wk (OR = 2.2, P < 0.030), and presence of VSD (OR = 3.8, P < 0.001) as independent predictors of in-hospital mortality. Children's general hospital and children's unit in a general hospital were found to have a lower mortality rate compared with not identified as a children's hospital after excluding immediate transfers (P = 0.008). CONCLUSIONS: BW < 1500 g, operation within 24 h, GA < 28 wk, and presence of VSD are the factors that predict higher mortality in EA/TEF population. Despite dealing with more complicated cases, children's general hospital and children's unit in a general hospital were able to achieve a lower mortality rate than not identified as a children's hospital.

Long-gap esophageal atresia: traction-growth and anastomosis - before and beyond.

Long-gap esophageal atresia (LGEA) is still a major surgical challenge. Options for esophageal reconstruction include the use of native esophagus or esophageal replacement with stomach, colon, or small intestine. Nonetheless, there is a consensus among most pediatric surgeons that the preservation of the native esophagus is associated with better postoperative outcomes. Thus, every effort should be made to conserve the native esophagus. The present study is aimed at critically reporting our experience focused on a standardized protocol based on the preoperative assessment of the gap in all cases and reviewing the present literature because no consensus is available regarding many aspects of LGEA (from definition to treatment). All newborn infants treated since 1995 for esophageal atresia (EA), regardless of type, were included in the present study. Identification of LGEA patients (gap ≥3 vertebral bodies) was performed based on preoperative esophageal gap measurement. The selected patients were grouped based on EA type (A/B vs. C/D) and whether they were referred from an outside institution or not. Postoperative outcome was compared. Statistical analysis was performed with the Fisher's exact test and Mann-Whitney test as appropriate, with P < 0.05 considered statistically significant. Two hundred and nineteen patients have been consecutively treated between 1995 and 2012 with the following EA subtypes: type: A 25 (11.4%); B 6 (2.7%); C 182 (83.1%); D 3 (1.4%); E 3 (1.4%). Fifty-seven patients (26%) were classified as LGEA: type A-B, 31 (54.4%); type C-D, 26 (45.6%). Twenty seven (47%) of these patients were referred after at least one failed attempt at esophageal correction: type A-B, 15 (55%); type C-D, 12 (45%). Only one patient ultimately required esophageal substitution, with an overall survival rate of 94%. A standardized perioperative protocol enhances the possibility of preserving the native esophagus in cases of LGEA. Gap measurement can be accurately defined before surgery in all patients with EA. Esophageal anastomosis (either immediate or delayed repair) is almost always feasible; esophageal substitution should only be considered after a rigorous attempt at achieving end-to-end esophageal anastomosis.

Formic acid poisoning in a tertiary care center in South India: A 2-year retrospective analysis of clinical profile and predictors of mortality.

BACKGROUND: Formic acid (FA), a common industrial compound, is used in the coagulation of rubber latex in Kerala, a state in southwestern India. Easy accessibility to FA in this region makes it available to be used for deliberate self-harm. However, the literature on intentional poisoning with FA is limited. STUDY OBJECTIVES: To determine the patterns of presentation of patients with intentional ingestion of FA and to find the predictors of mortality. A secondary objective was to find the prevalence and predictors of long-term sequelae related to the event. METHODS: We performed a 2-year chart review of patients with acute intentional ingestion of FA. Symptoms, signs, outcomes and complications were recorded, and patients who survived the attempt were followed-up by telephone or personal interview to identify any complications after their discharge from the hospital. RESULTS: A total of 302 patients with acute formic acid ingestion were identified during the study period. The mortality rate was 35.4% (n = 107). Bowel perforation (n = 39), shock (n = 73), and tracheoesophageal fistula (n = 4) were associated with 100% mortality. Quantity of FA consumed (p < 0.001), consuming undiluted FA (p < 0.001), presenting symptoms of hypotension (p < 0.001), respiratory distress (p < 0.001), severe degree of burns (p = 0.020), hematemesis (p = 0.024), complications like metabolic acidosis (p < 0.001) and acute respiratory distress syndrome (p < 0.001) were found to have significant association with mortality. The prevalence of esophageal stricture (n = 98) was 50.2% among survivors and was the most common long-term sequela among the survivors. Stricture was significantly associated with hematemesis (p < 0.001) and melena (p < 0.001). CONCLUSION: This study highlights the magnitude and ill-effects of self-harm caused by a strong corrosive, readily available due to very few restrictions in its distribution. Easy availability of FA needs to be curtailed by enforcing statutory limitations in this part of the world. Patients with hematemesis or melena after FA ingestion may be referred for early dilatation therapy in a setting where emergency endoscopic evaluation of all injured patients is not practical.

Combined airway and oesophageal stenting in malignant airway-oesophageal fistulas: a prospective study.

Malignant airway-oesophageal fistulas (AEF) are a serious complication of advance oesophageal or lung cancer. The aim of this study was to assess the quality of life before and after stent insertion, and to examine the role of treatment and location of AEF as factors influencing survival in AEF patients managed with airway and/or oesophageal stent insertion. 112 patients with AEF were included prospectively. 83 (74%) patients had advanced lung cancer and 29 (26%) patients had oesophageal cancers. Airway stents were inserted in 65 (58%) patients, oesophageal stents in 37 (33%) patients, and both airway and oesophageal stents in 10 (9%) patients. Seven (6%) patients developed respiratory failure and required transient ventilator support in the intensive care unit (four patients with airway stenting, two patients with double stents and one patient in the oesophageal stenting group). None of the patients developed stent migration or needed stent repositioning. Overall, mean survival was 236.6 days (airway stent 219.1 days, oesophageal stent 262.8 days and combined airway-oesophageal stent 252.9 days). Backward, stepwise regression revealed the site of stent placement (airway and/or oesophagus; p < 0.028), exact location of the fistula in airway (p = 0.011) and additional treatment with chemotherapy and/or radiation (p < 0.001) as independent risk factors predicting increased survival. The mean quality of life score (QoL) was 81 prior to stent insertion and 72 post-stent insertion (p < 0.001). Airway and/or oesophageal stent insertion provides an effective approach to improve the QoL in patients with malignant AEF.

Pre- and postnatal diagnosis and outcome of fetuses and neonates with esophageal atresia and tracheoesophageal fistula.

OBJECTIVES: Clinical symptoms and ultrasound signs during pregnancy could suggest the presence of esophageal atresia (EA). However, most often EA is diagnosed postnatally. The aim of our study is to evaluate the course and outcome for prenatally and postnatally diagnosed EA. In addition, we studied the outcome of isolated versus nonisolated EA. METHODS: In a retrospective data analysis, ultrasound characteristics, maternal and neonatal variables as well as clinical outcome were compared for fetuses/neonates with prenatal (n = 30) or postnatal (n = 49) diagnosis of EA. Clinical outcome in terms of morbidity and mortality of isolated EA was compared with that of EA complicated by chromosomal or structural anomalies. RESULTS: Prenatally diagnosed children were born 2 weeks earlier than postnatally diagnosed children (36.4 weeks vs 38.2 weeks; P = 0.02). The former had higher mortality rates (30 vs 12%; P = 0.05) and more associated anomalies (80 vs 59%; P = 0.04). In both subsets, there was a high morbidity rate in the survivors (not significant). Nonisolated EA was associated with greater occurrence of polyhydramnios (53 vs 27%; P = 0.04) and higher mortality rate (28 vs 0%; P = 0.002). CONCLUSIONS: Mortality was significantly higher in prenatally diagnosed infants and in infants with additional congenital anomalies. Isolated EA is associated with good outcome.

Early and long term outcome in children with esophageal atresia treated over the last 22 years.

BACKGROUND: The survival of infants born with esophageal atresia (EA) is > 90% at present. The purpose of this study was to evaluate early complications and long term outcome in children with EA treated at our institution. METHODS AND PATIENTS: Retrospective analysis of 111 children with EA undergoing repair of EA or tracheoesophageal fistula (TEF). Assessment of early and intermediate complications as well as long term morbidity and mortality. RESULTS: Primary anastomosis was performed in 90 (81%) and secondary anastomosis in 7 patients (6%). Gastric transposition was carried out in 14 children (13%). The postoperative mortality was 14/111 (12.6%) and could be estimated by the Spitz classification. At the age of 10 years, 33 patients (72%) were swallowing without problems, 39 children (85%) were eating at least most of the time with pleasure but 19 children (41%) had a body weight less than the 25 (th) percentile. Staged repair by gastric transposition resulted in the least amount of motility dysfunction. Long-term respiratory morbidity was high. CONCLUSION: The survival of children with EA has improved in the last two decades. For risk assessment the Spitz' classification is valid. Long term gastrointestinal and respiratory morbidity remains high. In children with long-gap EA gastric transposition performed as a staged procedure has satisfactory results and seems superior to techniques preserving the native esophagus.

Nationwide analysis of mortality and hospital readmissions in esophageal atresia.

PURPOSE: The purpose of this study is to identify determinants of mortality and hospital readmission in infants born with esophageal atresia ± tracheoesophageal fistula. METHODS: The Nationwide Readmissions Database (2010-2014) was queried for newborns with a diagnosis of esophageal atresia. Outcomes included mortality and readmissions at 30-day and 1-year. RESULTS: 3157 patients were identified, of which 54% were male. 81% had an additional congenital anomaly, and 35% had VACTERL association. Overall mortality at index hospitalization was 11% (n = 360) and was significantly higher with additional congenital anomalies (13%), VACTERL (19%), and Spitz classification II/III (18%) vs. isolated esophageal atresia/tracheoesophageal fistula (4%), all p < 0.001. After esophageal atresia repair (n = 2179), 10% (n = 212) were readmitted within 30 days and 26% (n = 563) within 1 year, with 17% admitted to different hospitals. Common diagnoses during readmission were GERD (54%), infections (42%), failure to thrive (17%), tracheomalacia (14%), and esophageal stricture (10%). Unplanned readmissions accounted for 85% of readmissions. A large number underwent operative procedures, most commonly esophageal dilation (17%) and fundoplication/gastrostomy (12%). CONCLUSION: Our study has uncovered a high likelihood of complications and unplanned readmission within the first year of life for newborns with esophageal atresia. Coordinated multidisciplinary care may help to decrease unnecessary readmissions and improve outcomes in this vulnerable population. TYPE OF STUDY: Retrospective comparative analysis. LEVEL OF EVIDENCE: Level III.

Fiberoptic bronchoesophagoscopy-assisted evaluation and prognostic factor analysis in children with congenital esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are serious congenital anomalies with high morbidity and mortality. Diagnostic and therapeutic fiberoptic endoscopy has been used in children to evaluate and manage trachea-esophageal anomalies. This study aimed to evaluate the prognostic factors and the role of fiberoptic bronchoesophagoscopy (FB) in managing children with EA and TEF. METHODS: From 2000 to 2017, hospitalized children with suspected EA and TEF were enrolled in the study. All associated medical records were retrospectively reviewed. Basic characteristics, diagnoses, age of surgical reconstruction, FB findings, associated anomalies, and survival durations were reviewed. Prognostic factors associated with the patients' mortality were analyzed. RESULTS: A total of 33 children were enrolled, and 91% of them were type C. The median age at the time of hospitalization was 26 days (range, birth to 9 years), including 20 (61%) low-birth-weight infants and 26 (79 %) referred patients. FB was performed in patients preoperatively (39%) and postoperatively (96.8%). Among them, 28 patients (85%) had associated anomalies, including 17 (52%) cardiac and 23 (70%) airway anomalies. The median age of 31 patients who underwent surgical reconstruction was 3 (range, 0-39) days. Esophageal anastomotic stricture (21/31, 67.7%) was the most common postsurgical complication. Twenty-three patients (74.2%) received postoperative FB-guided interventions, including balloon dilatation, laser therapy, and stent implantation. Among the 9 mortality cases, the median age at death was 270 (range, 4-3246) days. Significant factor associated with mortality was delayed (> 48 h old) or no surgical reconstruction (p = 0.030). CONCLUSION: Delayed (>48-hour old) or no surgical reconstruction was significantly related to mortality in children with congenital EA and TEF. Preoperative and postoperative FB evaluations helped to facilitate diagnoses and nonsurgical managements and resolve the patients' tracheoesophageal problems.

Comparative study of different treatments for malignant tracheoesophageal/bronchoesophageal fistulae.

The aim of this study is to compare the survival time and quality of life (QOL) of patients who have received different treatment for tracheoesophageal/bronchoesophageal fistula. Between January 2003 and December 2007, 35 patients with malignant tracheoesophageal/bronchoesophageal fistula were recorded as the control group, gastrostomy group, and stenting group, respectively, according to the treatments they chose. Two weeks after the treatment, European Organization for Research and Treatment of Cancer Quality of Life Core 30 Questionnaire (QLQ-C30), Quality of Life Questionnaire-esophageal module (QLQ-OES18), and a respiratory symptom-related QOL index are employed to assess QOL of these patients. There is no significant difference in survival time and constituent ratio of death reason among groups. Except for eight patients who died within 2 weeks after the treatment, all other 27 patients returned back the questionnaires. As compared to the control group, patients in the gastrostomy group gained a low score in emotional function and financial situation, while patients in the stenting group had lower scores in financial problems and seven respiratory and eating-related symptoms. In contrast with the gastrostomy group, patients in stenting group had higher scores in emotional and social functions, and lower scores in six respiratory and eating-related symptoms. With patients' QOL considered, the self-expandable coated stenting should be the first choice of therapy for malignant tracheoesophageal/bronchoesophageal fistula, whereas gastrostomy should be kept from use.

Factors affecting survival in patients with oesophageal atresia and tracheo-oesophageal fistula.

OBJECTIVE: To evaluate the various factors affecting survival in babies with oesophageal atresia and tracheo-oesophageal fistula. DESIGN: Descriptive study. SETTING: The study was carried out at the Department of Paediatric Surgery, The Children's Hospital, Pakistan Institute of Medical Sciences (PIMS), Islamabad from March 2004 to March 2005. PATIENTS AND METHODS: All neonates with oesophageal atresia (EA) and tracheo-oesophageal fistula (TEF) during the study period were included in the study. Patients having isolated EA were excluded. A total of 80 patients were included in the study. Patients were received from the emergency department, OPD and Neonatal ICU. Diagnosis was confirmed by passing a radio opaque orogastric tube. Investigations were done to look for other associations. After stabilisation, right thoracotomy was performed, fistula was ligated and divided. An attempt was made to do a primary oesophago-oesopahgostomy. Nasogastric feeding was started on 2nd post-operative day. A contrast oesophagogram was performed on the 7th postoperative day and having ruled out leak, oral feeding was started. RESULTS: Out of the total, 33 (41%) survived and 47 (58%) patients died. Out of 47 deaths 20 (25%) died before surgery and 27 (34%) died after surgery. Mean follow up period was 6 months. Sixteen (20%) patients had anastomotic leak, 24 (30%) had anastomotic stricture, and 64 (80%) patients had postoperative pneumonia. CONCLUSION: We conclude that proper antenatal check ups will detect the problem early, avoid home deliveries and hence improve survival. Pneumonitis and septicaemia significantly affect survival. Availability of ICU is one of the main determinants of survival. The likely cause of high mortality rate in pre-operative patients in our series is non-availability ofNICU due to limited space in our setup.

State of Play: Eight Decades of Surgery for Esophageal Atresia.

AIM: Surgical expertise and advances in technical equipment and perioperative management have led to enormous progress in survival and morbidity of patients with esophageal atresia (EA) in the last decades. We aimed to analyze the available literature on surgical outcome of EA for the past 80 years. MATERIALS AND METHODS: A PubMed literature search was conducted for the years 1944 to 2017 using the keywords "esophageal/oesophageal atresia," "outcome," "experience," "management," and "follow-up/follow up." Reports on long-gap EA only, non-English articles, case reports, and reviews without original patient data were excluded. We focused on mortality and rates of recurrent fistula, leakage, and stricture. RESULTS: Literature search identified 747 articles, 118 manuscripts met the inclusion criteria. The first open end-to-end anastomosis and fistula ligation was reported in 1941. Thoracoscopic fistula ligation and primary anastomosis was performed first in 2000. Reported mortality rate decreased from 100% before 1941 to 54% in 1950 to 1959, 28% in 1970 to 1979, 16% in 1990 to 1999, and 9% nowadays. Rates of recurrent fistula varied over time between 4 and 9%. Leakage rate remained stable between 11 and 16%. However, stricture rate increased from 25 to 38%. CONCLUSION: Including a full range of articles reflecting the heterogeneity of EA, mortality rate significantly decreased during the course of 80 years. Along with the decrease in mortality, there is a shift to the importance of major postoperative complications and long-term morbidity regardless of surgical technique.

Relationships between hospital and surgeon operative volumes and outcomes of esophageal atresia/tracheoesophageal fistula repair.

PURPOSE: Most pediatric surgeons perform <2 esophageal atresia and tracheoesophageal fistula (EA/TEF) repairs annually. We aimed to determine whether higher surgeon and hospital volumes are associated with better outcomes after EA/TEF repair. METHODS: Neonates with a diagnosis and repair of EA/TEF at their index hospital admission in the Pediatric Health Information System from 1/2000 to 9/2015 were included. For each patient, hospital and surgeon operative volumes were defined as the number of EA/TEF cases treated in the previous 365 days. Propensity score weighting was used to estimate relationships between operative volumes and rates of in-hospital mortality, readmission within 30 days, and readmission, reoperation, and dilation within one year. RESULTS: Among 3085 patients, lower birth weight, earlier gestational age, the presence of congenital heart disease, and certain other anomalies were associated with higher mortality. In risk-adjusted analyses, there were no significant differences in mortality or any other outcome based on hospital or surgeon volume alone or when comparing low- or high-volume surgeons practicing at low- or high-volume hospitals. CONCLUSIONS: Neither surgeon nor hospital volume significantly impacted outcomes after EA/TEF repair. Our findings imply that selective referral and pediatric surgeon subspecialization in EA/TEF may not translate to improved outcomes. TYPE OF STUDY: Retrospective comparative study LEVEL OF EVIDENCE: Level III.

Outcomes of multi-gestational pregnancies affected by esophageal atresia - tracheoesophageal fistula.

BACKGROUND: Contemporary outcomes of infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) from multi-gestational pregnancies compared to those of singleton pregnancies have not been reported. METHODS: A single-center retrospective review of EA/TEF patients born from 1999 to 2013 was performed. Patient demographics, gestational age (GA), birth weight, associated anomalies, requirement for gastrostomy tube and mortality were reviewed. RESULTS: Singleton EA/TEF patients outnumbered those from multi-gestational pregnancies nearly 10:1 (214 vs 22 patients). EA/TEF patients from multi-gestational pregnancies were more likely to be premature (77% vs. 32%), have lower birth weight (mean 1766 g vs. 2695 g), have associated duodenal atresia (18% vs. 6%) and require gastrostomy tube (41% vs. 33%) for feeding challenges compared to EA/TEF singletons. Mortality was also significantly greater for multi-gestational EA/TEF patients compared to singleton EA/TEF patients (18% vs. 6%). CONCLUSION: EA/TEF infants from multi-gestational pregnancies have greater clinical complexity and mortality than singleton EA/TEF patients. Parents of EA/TEF multi-gestational infants should be appropriately counseled and supported.

Neurodevelopmental outcomes of infants with esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is a complex disorder, and most outcome data are confined to mortality and feeding-related morbidities. Our objective was to examine mortality, growth and neurodevelopmental outcomes in a large recent cohort of infants with EA/TEF. METHODS: Single center study of EA/TEF infants referred from January 2000 to December 2015. Data collected included associated defects, neonatal morbidity and mortality and growth and neurodevelopmental outcomes at age 12-36months. Multiple regression analysis was used to determine variables associated with adverse outcome. RESULTS: Of the 253 infants identified, 102 infants (40%) were preterm. Overall mortality was 8.3%, the majority from major cardiac malformations (p<0.001) Neurodevelopmental assessments (n=182) showed that 76% were within normal, while some delay was seen in 24%, most often in expressive and receptive language. Nine infants had hearing impairment and 5 had visual impairment. Gastrostomy tubes were required in 47 patients and 15% continued to have weight growth velocities less than the 10th centile. A number of specialist interventions were required, Speech/Language being frequent. CONCLUSION: Mortality in EA/TEF is primarily related to concomitant anomalies, especially cardiac. Multidisciplinary follow up is important for early identification and intervention for growth failure and developmental delay. TYPE OF STUDY: Retrospective study LEVEL OF EVIDENCE: Level II.